The coexistence of systemic lupus erythematosus (SLE) and ANCA-associated vasculitis (AAV) as an overlapping syndrome is not common. Here, we report a case of a 33-year-old woman, with recent SLE diagnosis due to skin, kidney, articular, and immunologic compromise, in whom a chest CT scan showed bilateral nodules, consolidations, and tree-in-bud pattern; thoracoscopic lung biopsy revealed diffuse non-caseating granulomas, without other features of sarcoid, organizing pneumonia, or hypersensitivity pneumonitis with high positive p-ANCA titers. Overlap between SLE and AAV was a possible explanation for lupus granulomatous pneumonitis, and for this reason, a multidisciplinary meeting was held to evaluate complex patients with interstitial lung diseases patients.